Seroatlas · Human Serome Atlas

MT-CO1

Cytochrome c oxidase subunit 1

Also known as: COI, COX1, COX1_HUMAN, MTCO1

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
P00395
Gene
MT-CO1
Ensembl
ENSG00000198804
Chromosome
MT
Canonical length
513 aa
Protein class
Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted membrane proteins, Transporters
Subcellular location
Mitochondria,End piece

OverviewNCBI Gene

Contributes to cytochrome-c oxidase activity. Predicted to be involved in aerobic respiration; positive regulation of vasoconstriction; and respiratory electron transport chain. Located in mitochondrial membrane. Part of respiratory chain complex IV. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

513 residues, UniProt reviewed canonical sequence.

>P00395|MT-CO1
     1  MFADRWLFST NHKDIGTLYL LFGAWAGVLG TALSLLIRAE LGQPGNLLGN DHIYNVIVTA
    61  HAFVMIFFMV MPIMIGGFGN WLVPLMIGAP DMAFPRMNNM SFWLLPPSLL LLLASAMVEA
   121  GAGTGWTVYP PLAGNYSHPG ASVDLTIFSL HLAGVSSILG AINFITTIIN MKPPAMTQYQ
   181  TPLFVWSVLI TAVLLLLSLP VLAAGITMLL TDRNLNTTFF DPAGGGDPIL YQHLFWFFGH
   241  PEVYILILPG FGMISHIVTY YSGKKEPFGY MGMVWAMMSI GFLGFIVWAH HMFTVGMDVD
   301  TRAYFTSATM IIAIPTGVKV FSWLATLHGS NMKWSAAVLW ALGFIFLFTV GGLTGIVLAN
   361  SSLDIVLHDT YYVVAHFHYV LSMGAVFAIM GGFIHWFPLF SGYTLDQTYA KIHFTIMFIG
   421  VNLTFFPQHF LGLSGMPRRY SDYPDAYTTW NILSSVGSFI SLTAVMLMIF MIWEAFASKR
   481  KVLMVEEPSM NLEWLYGCPP PYHTFEEPVY MKS

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against MT-CO1 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Other membrane
Secreted
No
Transmembrane segments
12
Mean surface accessibility (rSASA)
0.21
Highest tissue expression
265,339 nTPM

Expression across tissuesHPA

Tissue

  • heart muscle: 265,339 nTPM
  • tongue: 172,333 nTPM
  • skeletal muscle: 137,864 nTPM
  • hippocampal formation: 128,484 nTPM
  • basal ganglia: 128,239 nTPM
  • amygdala: 128,136 nTPM

Single-cell type

  • enterocytes: 89,897 nCPM
  • colonocytes: 61,582 nCPM
  • hepatocytes: 54,247 nCPM
  • paneth cells: 38,566 nCPM
  • goblet cells: 37,002 nCPM
  • esophageal apical cells: 35,092 nCPM

Immune cell

  • classical monocyte: 9,761 nTPM
  • non-classical monocyte: 5,806 nTPM
  • naive B-cell: 5,586 nTPM
  • memory B-cell: 5,393 nTPM
  • basophil: 5,335 nTPM
  • intermediate monocyte: 4,911 nTPM

Brain region

  • choroid plexus: 163,846 nTPM
  • thalamus: 162,758 nTPM
  • medulla oblongata: 149,673 nTPM
  • amygdala: 147,754 nTPM
  • pons: 142,295 nTPM
  • cerebral cortex: 136,220 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about MT-CO1.

Disease | AllUniProt

Conditions MT-CO1 is implicated in, by any mechanism.

  • Leber hereditary optic neuropathy (LHON) MIM:535000
  • Mitochondrial complex IV deficiency (MT-C4D) MIM:220110
  • Recurrent myoglobinuria mitochondrial (RM-MT) MIM:550500
  • Deafness, sensorineural, mitochondrial (DFNM) MIM:500008
  • Colorectal cancer (CRC) MIM:114500

Disease | GeneticClinVar

21 pathogenic / likely-pathogenic of 238 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

  • Cytochrome c oxidase subunit I
  • Cytochrome c oxidase, subunit I, copper-binding site
  • Cytochrome c oxidase-like, subunit I domain
  • Cytochrome c oxidase subunit I domain
  • Cytochrome c oxidase-like, subunit I superfamily
  • Cytochrome C and Quinol oxidase polypeptide I

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of MT-CO1 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads MT-CO1 as an antibody target. Whether an autoantibody or antibody against MT-CO1 could matter depends on whether native MT-CO1 is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

MT-CO1 is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label MT-CO1 as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/MT-CO1. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

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